Neurobiology of Disease The Induction Levels of Heat Shock Protein 70 Differentiate the Vulnerabilities to Mutant Huntingtin among Neuronal Subtypes
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چکیده
Kazuhiko Tagawa,1 Shigeki Marubuchi,1,2 Mei-Ling Qi,1,3 Yasushi Enokido,1 Takuya Tamura,1 Reina Inagaki,1 Miho Murata3 Ichiro Kanazawa,4 Erich E. Wanker,5 and Hitoshi Okazawa1,3,6 1Department of Neuropathology, Medical Research Institute and 21st Century Center of Excellence Program for Brain Integration and Its Disorders, Tokyo Medical and Dental University, Tokyo 113-8510, Japan, 2Toyama Chemical Company, Toyama 930-8508, Japan, 3PRESTO, Japan Science and Technology Agency, Kawagoe 332-0012, Japan, 4National Center for Neurology and Psychiatry, Kodaira 187-8502, Japan, and 5Max-Delbrück Center for Molecular Medicine, D-13125 Berlin, Germany
منابع مشابه
The induction levels of heat shock protein 70 differentiate the vulnerabilities to mutant huntingtin among neuronal subtypes.
The reason why vulnerabilities to mutant polyglutamine (polyQ) proteins are different among neuronal subtypes is mostly unknown. In this study, we compared the gene expression profiles of three types of primary neurons expressing huntingtin (htt) or ataxin-1. We found that heat shock protein 70 (hsp70), a well known chaperone molecule protecting neurons in the polyQ pathology, was dramatically ...
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Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterized by the progressive loss of motor neurons leading to paralysis and death. Mutations of the human Cu/Zn superoxide dismutase (SOD1) are found in some cases of familial ALS (fALS). Recent evidences suggest the accumulation of intracellular calcium is one of the primary mechanisms of motor neuronal degeneration. In th...
متن کاملHeat shock protein 70 protects motor neuronal cells expressing mutant Cu/Zn superoxide dismutase (SOD1) against altered calcium homeostasis
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterized by the progressive loss of motor neurons leading to paralysis and death. Mutations of the human Cu/Zn superoxide dismutase (SOD1) are found in some cases of familial ALS (fALS). Recent evidences suggest the accumulation of intracellular calcium is one of the primary mechanisms of motor neuronal degeneration. In th...
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Neuronal loss and intraneuronal protein aggregates are characteristics of Huntington's disease (HD), which is one of 10 known neurodegenerative disorders caused by an expanded polyglutamine [poly(Q)] tract in the disease protein. N-terminal fragments of mutant huntingtin produce intracellular aggregates and cause toxicity. Several studies have shown that chaperones suppress poly(Q) aggregation ...
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Huntington ́s disease, one of the so-called poly-glutamine diseases, is a dominantly inherited movement disorder characterized by formation of cytosolic and nuclear inclusion bodies and progressive neurodegeneration. Recently, we have shown that Bcl-2-associated athanogene-1 (BAG1), a multifunctional co-chaperone, modulates toxicity, aggregation, degradation and subcellular distribution in vitro...
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تاریخ انتشار 2007